A novel mutation in ACADVL causing very long-chain acyl-coenzyme-A dehydrogenase deficiency in a South Asian pediatric patient: a case report and review of the literature [PDF]
Journal of Medical Case Reports, 2021 Background Very long-chain acyl-coenzyme-A dehydrogenase deficiency is a rare, severe life-threatening metabolic disorder of mitochondrial fatty acid oxidation, caused by mutations in ACADVL gene.
Visvalingam Arunath +5 more
doaj +3 more sources
Progress of the acyl-Coenzyme A thioester hydrolase family in cancer [PDF]
Frontiers in OncologyIn recent years, the acyl-Coenzyme A thioester hydrolase family (ACOTs) has received wide attention as a key link in lipid metabolism. This family is a class of enzymes that catalyze the hydrolysis of fatty acyl-Coenzyme A, disrupting the thioester bond ...
Lu Bai +4 more
doaj +4 more sources
Glycogen storage disease type Ia misdiagnosed as multiple acyl-coenzyme A dehydrogenase deficiency by mass spectrometry [PDF]
Frontiers in Pediatrics, 2022 ObjectiveTo report a case of glycogen storage disease (GSD) type Ia misdiagnosed as multiple acyl-coenzyme a dehydrogenase deficiency (MADD) by mass spectrometry.MethodsA 7 months old boy was admitted to our hospital for elevated transaminase levels ...
Juan Du +5 more
doaj +2 more sources
An update on the therapeutic implications of long-chain acyl-coenzyme A synthetases in nervous system diseases [PDF]
Frontiers in Neuroscience, 2022 Long-chain acyl-coenzyme A synthetases (ACSLs) are a family of CoA synthetases that activate fatty acid (FA) with chain lengths of 12–20 carbon atoms by forming the acyl-AMP derivative in an isozyme-specific manner.
Zhimin Wu +6 more
doaj +2 more sources
Anesthesia management in a patient with very long-chain acyl-Coenzyme A dehydrogenase deficiency [PDF]
JA Clinical Reports, 2020 Background In a patient with very long-chain acyl-Coenzyme A dehydrogenase (VLCAD) deficiency, metabolism of fatty acids is impaired and a supply of alternative energy is limited when glucose level is insufficient on starvation.
Haruyuki Yuasa +5 more
doaj +2 more sources
Effects of ligand binding on the energy landscape of acyl-CoA-binding protein [PDF]
Biophys. J. 119 (2020) 1821-1832, 2021 Binding of ligands is often crucial for function yet the effects of ligand binding on the mechanical stability and energy landscape of proteins are incompletely understood. Here we use a combination of single-molecule optical tweezers and MD simulations to investigate the effect of ligand binding on the energy landscape of acyl-coenzyme A (CoA) binding
arxiv +1 more source
The pathophysiology of leg cramping during dialysis and the use of carnitine in its treatment
Physiological Reports, 2021 Leg cramping is a common side effect of hemodialysis, and this is frequently treated by the administration of carnitine, but this is not effective in every patient. Alkalosis is a key component of the etiology of leg cramping during hemodialysis sessions.
Akira Takahashi
doaj +1 more source
mBio, 2023 Members of the genus Mesorhizobium, which are core components of the rhizosphere and specific symbionts of legume plants, possess genes for acyl-homoserine lactone (AHL) quorum sensing (QS).
Zehui Suo +4 more
doaj +1 more source
An acyl-CoA synthetase in Mycobacterium tuberculosis involved in triacylglycerol accumulation during dormancy. [PDF]
PLoS ONE, 2014 Latent infection with dormant Mycobacterium tuberculosis is one of the major reasons behind the emergence of drug-resistant strains of the pathogen worldwide.
Jaiyanth Daniel +2 more
doaj +1 more source
In Search of Small Molecules That Selectively Inhibit MBOAT4
Molecules, 2021 Ghrelin is a 28-residue peptide hormone produced by stomach P/D1 cells located in oxyntic glands of the fundus mucosa. Post-translational octanoylation of its Ser-3 residue, catalyzed by MBOAT4 (aka ghrelin O-acyl transferase (GOAT)), is essential for ...
Emily S. Murzinski +7 more
doaj +1 more source